中文摘要：

目的探究晚发型视神经脊髓炎谱系疾病（LONMOSD）患者的临床特征。方法回顾性分析2010年1月—2015年5月收治的61例LONMOSD患者的临床资料。结果 （1）61例LONMOSD患者,起病年龄为57（53,63）岁,男女比例1∶3.7。32例（52.5%）以横贯性脊髓炎起病,16例（26.2%）以视神经炎起病。51例（83.6%）表现为复发-缓解病程。头部磁共振检查异常者40例（65.6%）,脊髓磁共振检查胸髓受累者最多（39.3%）。（2）水通道蛋白4（AQP-4）抗体阳性组与阴性组之间临床特征比较差异无统计学意义。（3）Spearman相关分析显示,急性期及缓解期扩展功能障碍状况量表（EDSS）评分与血清AQP-4抗体水平呈正相关（rs分别为0.389、0.380,均P〈0.01）,而脊髓平均受累节段数及发作次数与血清AQP-4抗体水平无明显相关性（rs分别为0.146、0.096,均P〉0.05）。结论 LONMOSD多以横贯性脊髓炎起病、胸髓最常受累、常合并脑部病变。AQP-4抗体水平与疾病的严重程度有一定相关性。

英文摘要：

Objective To explore the clinical features of late-onset neuromyelitis optica spectrum disorders（LON-MOSD）. Methods A retrospective analysis was performed to evaluate 61 patients with LONMOSD admitted to our hospitalfrom January 2010 to May 2015. Results（1） The median age at onset was 57（53, 63） years, male/female was 1∶3.7. Thirty-two patients（52.5%） had transverse myelitis（TM） and 16 patients（26.2%） had optic neuritis（ON） at the disease onset. Fifty-one patients（83.6%） experienced recurrent attacks. Forty patients（65.6%） showed abnormal brain magnetic resonance imag-ing（MRI）. Spinal cord MRI showed more frequently present in thoracic regions（39.3%）.（2） There were no significant differ-ences in clinical features between AQP-4 seropositive and seronegative groups.（3） By Spearman analysis, it was obviousthat EDSS scores at acute phase and remission were positively correlated to AQP-4 antibody levels（rs=0.389, P 0.01; rs=0.380, P 0.01）. But there was no correlation between the mean segments of spinal cord and relapse rates with AQP-4 anti-body levels（rs=0.146, P 0.05; rs=0.096, P 0.05）. Conclusion LONMOSD patients are more prone to present with TM atonset and have more lesions in thoracic spinal cord and brain. The AQP-4 antibody titres can indicate the severity of diseasein acute phase.